Download Left main bronchus compression due to main pulmonary artery

CASE REPORT
Left main bronchus compression due to main pulmonary artery
dilatation in pulmonary hypertension: two case reports
Shareen K. Jaijee,1,2 Ben Ariff,3 Luke Howard,4 Declan P. O’Regan,2 Wendy Gin-Sing,4 Rachel Davies,4 J. Simon R. Gibbs4
1
University of Sydney, Camperdown, New South Wales, Australia; 2MRC Clinical Sciences Centre, Imperial College London, Hammersmith Hospital Campus,
London, United Kingdom; 3Department of Imaging, Imperial College London, Hammersmith Hospital, London, United Kingdom; 4Pulmonary Hypertension
Service, Imperial College London, Hammersmith Hospital, London, United Kingdom
Abstract: Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures.
Left main coronary artery and left recurrent laryngeal nerve compression have been described. Tracheobronchial compression from
pulmonary arterial dilatation is rare in adults, and there are no reports in the literature of its occurrence in idiopathic pulmonary arterial
hypertension. Compression in infants with congenital heart disease has been well described. We report 2 cases of tracheobronchial compression: first, an adult patient with idiopathic pulmonary arterial hypertension who presents with symptomatic left main bronchus compression, and second, an adult patient with Eisenmenger ventricular septal defect and right-sided aortic arch, with progressive intermedius
and right middle lobe bronchi compression in association with enlarged pulmonary arteries.
Keywords: bronchus compression, pulmonary arterial dilatation, complications.
Pulm Circ 2015;5(4):723-725. DOI: 10.1086/683687.
Pulmonary arterial dilatation in the setting of pulmonary hypertension is a common finding.1 Extrinsic compression of the left
main coronary artery and the left recurrent laryngeal nerve due to
pulmonary arterial dilatation has been reported in the literature.2-5
While uncommon, tracheobronchial compression due to pulmonary arterial dilatation is a well-known complication in infants with
complex congenital heart disease and vascular rings.6 In absent
pulmonary valve syndrome, significant symptoms in early infancy
are commonly due to bronchial compression from dilated pulmonary arteries and an enlarged right atrium.7 In adults, respiratory
symptoms caused by tracheobronchial compression are most commonly due to aortic aneurysms in the setting of atherosclerosis.8
To our knowledge, there have been no reports of tracheobronchial compression in patients with idiopathic pulmonary arterial hypertension and pulmonary arterial dilatation. We present the first
report of an adult patient with severe idiopathic pulmonary arterial
hypertension and pulmonary arterial dilatation associated with symptomatic left main bronchus compression. In addition, we present a
case of adult congenital heart disease associated with pulmonary
arterial dilatation, with progressive, symptomatic bronchus intermedius and right middle lobe bronchus compression.
C AS E D E S C R I P T I O N
Patient A is a 73-year-old female who was diagnosed and commenced on treatment for idiopathic pulmonary arterial hyperten-
sion 11 years earlier. On diagnosis, she had a pulmonary arterial
systolic pressure of 98 mmHg, diastolic pressure of 40 mmHg,
mean pulmonary artery pressure of 67 mmHg, pulmonary capillary
wedge pressure of 8 mmHg, mean right atrial pressure of 10 mmHg,
cardiac index of 1.92 L/min/m2, and pulmonary vascular resistance
of 1,181 dyn-s/cm5 on right heart catheterization. She was noted to
have enlarged pulmonary arteries, 1.5 times the size of the aorta
on computed tomography (CT). Over time, she progressed from New
York Heart Association (NYHA) class II to class IV symptoms.
CT 10 years after diagnosis showed that the main pulmonary
artery abutted but did not compress the left main coronary artery.
One year later, she noted a voice change and bovine cough, suggesting vocal cord dysfunction. She was admitted to the hospital
with a lower respiratory tract infection and worsening shortness of
breath, which only partially resolved with antibiotics and then recurred. Because of progressive shortness of breath and sputum production, she had high-resolution CT that showed aneurysmal dilatation of the central pulmonary arteries. The right pulmonary artery
had increased in size to 91 mm in diameter and was associated with
left main bronchus compression (Figs. 1, 2).
Spirometry was not tolerated because of cough, and flow-volume
loops could not be recorded. Peak flow was severely reduced to an
average of 183 L/min from 3 attempts. Her treatment included
bosentan and sildenafil. She was subsequently referred to specialist
palliative care for symptom control and has continued on medical
therapy.
Address correspondence to Dr. J. Simon R. Gibbs, Pulmonary Hypertension Service, Hammersmith Hospital, London, United Kingdom. E-mail: s.gibbs
@imperial.ac.uk.
Submitted March 7, 2014; Accepted May 13, 2015; Electronically published November 4, 2015.
© 2015 by the Pulmonary Vascular Research Institute. All rights reserved. 2045-8932/2015/0504-0014. $15.00.
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| Left main bronchus compression due to pulmonary artery dilatation
Figure 1. Contrast-enhanced computed tomography of the chest
reconstructed in the coronal plane of patient A showing left main
bronchus compression (arrow) by an enlarged main pulmonary
artery.
Patient B is a 63-year-old male with Eisenmenger’s complex
associated with right-sided aortic arch, chronic obstructive pulmonary disease, and atrial fibrillation who had a 10-year history of increasing shortness of breath. Severe pulmonary hypertension with
a bidirectional shunt across the ventricular septal defect was confirmed on echocardiography, with an estimated right ventricular
systolic pressure of 116 mmHg. Over time, he progressed to NYHA
class IV symptoms, and his current treatment includes warfarin,
bosentan, and sildenafil.
For 2 years, he has had an increasing frequency of lower respiratory tract infections and has expectorated an eggcup full of gray-green
sputum every day. CT 3 years ago showed bronchus intermedius
and middle lobe bronchus compression with right middle lobe collapse. This progressed 2 years later with aneurysmal dilatation of
the right pulmonary artery to 51 mm with intrinsic thrombus, occlusion of the right middle lobe bronchus with consequent collapse
of the right middle lobe, and significant compression of the bronchus intermedius (Fig. 3). He has continued on medical therapy.
DISCUSSION
In contrast to adults, the cartilaginous, muscular, and elastic supports of the airway in infants are weak, making the tracheobronchial tree vulnerable to compression. The left main bronchus is
located between the descending aorta and the pulmonary artery,
making it susceptible to compression with dilation of these vessels.9,10 Acyanotic congenital heart disease including absent pulmonary valve syndrome and atrial septal defects causing pulmonary
arterial dilatation,9-13 right-sided aortic arch, double aortic arch, and
abnormal ligamentous arteriosus insertion,6 associated with pulmonary arterial dilatation and tracheobronchial compression, have
been reported.
Jaijee et al.
Pulmonary arterial dilatation is a common complication of severe pulmonary arterial hypertension and is not associated with
increased risk of death. Our review of the literature found 2 cases
of tracheobronchial compression from dilated pulmonary arteries
in adults. Symptomatic tracheobronchial compression was reported
in an 18-year-old female with newly diagnosed absent pulmonary
valve syndrome,14 and asymptomatic left bronchial compression was
reported in a 55-year-old male with chronic thromboembolic disease
and congenital pulmonary stenosis with consequent pulmonary arterial dilatation.15 Left main coronary artery compression and left
recurrent laryngeal nerve compression are otherwise well-reported
complications of pulmonary arterial dilatation.2-5
Vascular tracheobronchial compression syndromes rarely present in adulthood. The most common acquired vascular tracheobronchial compression syndrome in adults is due to compression
from aortic aneurysms in the setting of either atherosclerosis or
Marfan’s syndrome.8 In adults, CT is the investigation of choice and
avoids invasive bronchoscopy. Flow-volume curves may show flattening of the expiratory portion of the curve, suggesting variable
intrathoracic obstruction, and the chest x-ray may show a widened
upper mediastinum.
Treatment of tracheobronchial compression in infants and children has been successful with surgical decompression6,13,16 or endobronchial stenting.9 In adults, patients are likely to be unsuitable
for surgical intervention. In the 2 cases previously reported in the
literature, one patient died soon after diagnosis,14 and the other
managed with pulmonary vasodilators.15 Endobronchial stenting has
not been performed.
In conclusion, we present 2 patients with massive pulmonary
arterial dilatation causing tracheobronchial compression. Diagnosis was made on CT, with the clinical presentation of recurrent
lower respiratory tract infections in both patients. Large airway compression should be considered in patients with pulmonary arterial
Figure 2. Contrast-enhanced computed tomography image of patient A showing left main bronchus compression (arrow) by the
main pulmonary artery.
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Pulmonary Circulation
4.
5.
6.
7.
8.
9.
Figure 3. Contrast-enhanced computed tomography reconstructed
in the coronal plane of patient B showing right bronchus compression (arrow).
10.
hypertension associated with markedly enlarged pulmonary arteries
as a potential cause of worsening breathlessness.
11.
Source of Support: Nil.
12.
Conflict of Interest: None declared.
R E F E R E N CE S
13.
1. Badagliacca R, Poscia R, Pezzuto B, Papa S, Nona A, Mancone M,
Mezzapesa M, et al. Pulmonary arterial dilatation in pulmonary hypertension: prevalence and prognostic relevance. Cardiology 2012;121(2):
76–82.
2. Heikkinen J, Milger K, Alejandre-Lafont E, Woitzik C, Litzlbauer D,
Vogt J-F, Klußmann JP, Ghofrani A, Krombach GA, Tiede H.
Cardiovocal syndrome (Ortner’s syndrome) associated with chronic
thromboembolic pulmonary hypertension and giant pulmonary artery
aneurysm: case report and review of the literature. Case Rep Med 2012;
2012:230736.
3. Andjelkovic K, Kalimanovska-Ostric D, Djukic M, Vukcevic V,
Menkovic N, Mehmedbegovic Z, Topalovic M, Tesic M. Two rare con-
14.
15.
16.
Volume 5
Number 4
December 2015
| 725
ditions in an Eisenmenger patient: left main coronary artery compression and Ortner’s syndrome due to pulmonary artery dilatation. Heart
Lung 2013;42(5):382–386.
Fujiwara K, Naito Y, Higashiue S, Takagaki Y, Goto Y, Okamoto M,
Yoshida S, Sekii H, Tomobuchi Y. Left main coronary trunk compression by dilated main pulmonary artery in atrial septal defect. Report
of three cases. J Thorac Cardiovasc Surg 1992;104:449–452.
Lee J, Kwon HM, Hong BK, Kim HK, Kwon KW, Kim JY, Lee KJ,
et al. Total occlusion of left main coronary artery by dilated main
pulmonary artery in a patients with severe pulmonary hypertension.
Korean J Intern Med 2001;16:265–269.
Bove T, Demanet H, Casmir G, Viart P, Goldstein J, Deuvaert F.
Tracheobronchial compression of vascular origin: review of experience
in infants and children. J Cardiovasc Surg 2001;42:663–666.
Pinsky W, Nihill M, Mullins C, Harrison G, McNamara D. The absent pulmonary valve syndrome. Considerations of management. Circulation 1978;57:159–162.
Kanabuchi K, Noguchi N, Kondo T. Vascular tracheobronchial compression syndrome in adults: a review. Tokai J Exp Clin Med 2011;36:106–
111.
Saygili A, Aytekin C, Boyvat F, Barutcu O, Mercan S, Tokel K. Endobronchial stenting in a two-month-old infant with bronchial compression secondary to tetralogy of Fallot and absent pulmonary valve. Turk
J Pediatr 2004;46:268–271.
Berlinger N, Long CS, Foker J, Lucas RJ. Tracheobronchial compression in acynoatic congenital heart disease. Ann Otol Rhinol Laryngol
1983;92:387–390.
Corno A, Picardo S, Ballerini L, Gugliantini P, Marcelletti C. Bronchial
compression by dilated pulmonary artery. Surgical treatment. J Thorac
Cardiovasc Surg 1985;90:706–710.
Knauth AL, Marshall AC, Geva T, Jonas RA, Marx GR. Respiratory
symptoms secondary to aortopulmonary collateral vessels in tetralogy of
Fallot absent pulmonary valve syndrome. Am J Cardiol 2004;93(4):503–
505.
Talwar S, Sharma P, Choudhary SK, Kothari SS, Gulati GS, Airan B.
Le-Compte’s maneuver for relief of bronchial compression in atrial septal
defect. J Cardiac Surg 2011;26(1):111–113.
Lanjewar C, Shiradkar S, Agrawal A, Mishra N, Kerkar P. Aneurysmally
dilated major aorto-pulmonary collateral in tetralogy of Fallot. Indian
Heart J 2012;64(2):196–197.
Morjaria S, Grinnan D, Voelkel NF. Massive dilatation of the pulmonary artery in association with pulmonic stenosis and pulmonary hypertension. Pulm Circ 2012;2(2):256–257.
Sebening C, Jakob H, Tochtermann U, Lange R, Vahl C, Bodegom P,
Szabo G, et al. Vascular tracheobronchial compression syndromes—
experience in surgical treatment and literature review. Thorac Cardiovasc Surg 2000;48(3):164–174.
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All use subject to University of Chicago Press Terms and Conditions (http://www.journals.uchicago.edu/t-and-c).